Interrupted aortic arch: an eight-year experience

INTRODUCTION: Interrupted aortic arch (IAA) is a rare congenital heart defect with a high mortality rate in the neonatal period. Surgical correction of associated intracardiac anomalies can be performed in a one-stage (primary) or two-stage approach. OBJECTIVES: Case review of children with IAA operated in our center and to evaluate the surgical outcomes and the occurrence of complications. METHODS: A retrospective chart review of children operated from June 1998 to October 2006. RESULTS: Twelve children (nine girls and three boys) were operated. Nine patients had ventricular septal defect with septal malalignment, two had univentricular hearts and two had transposition of the great arteries. Primary correction was performed in eleven patients (aged between two and 38 days), including two Norwood procedures. There was no early mortality. The follow-up time ranged from 30 days to six years (median 2.6 years), with late mortality of 33%. Two children required reintervention for aortic arch restenosis, which was successfully treated by catheterization. DISCUSSION: Our experience is that early primary repair of IAA has low early and late mortality and is the method of choice most situations. Late mortality depends the severity of associated anomalies.